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29 Cards in this Set
- Front
- Back
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The mucinoses are a heterogenous group of disorders in which an abnormal amount of mucin accumulates in the skin either focally of diffusely. What is the difference between primary and secondary mucinoses? |
Primary: the mucin deposition is the major histologic feature and results in clinically distinctive lesions
Secondary: the mucin deposition is simply an associated finding (eg CTD, GA, neural tumors, hypertrophic scars) |
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What are the main glycosaminoglycans in the dermis? |
Dermatan sulfate Chondroitin sulfate Heparan sulfate Hyaluronic acid |
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Which GAG is NOT sulfated? |
Hyaluronic acid |
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When we talk about 'Cutaneous Mucinosis' or 'Lichen Myxedematosus', which GAGs are we talking about? |
Hyaluronic acid (remember this is non sulfated) |
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What are the primary GAGs in mucopolysaccharidosis (ie Hunter or Hurler syndrome)? |
Dermatan sulfate Heparan sulfate |
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Three stains that assist in confirming the presence of mucin in the epidermis? |
Alcian blue Toluidine blue Colloidal iron
BLUE, BLUE, IRON |
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Which primary cutaneous mucinoses are associated with:
Paraproteinemia?
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Scleromyxedema, scleredema |
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Which primary cutaneous mucinoses are associated with:
Diabetes? |
Scleredema |
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Which primary cutaneous mucinoses are associated with:
Thyroid disease? |
Pretibial myxedema, myxedema |
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DX? Clinical presentation? |
Scleromyxedema (Generalized lichen myxedematosus)
Clincally it is chronic and progressive as multiple waxy, 2-4mm, dome shaped or flat topped papules that can coalesce on dorsal hands, face (leonine facies), elbows, extensor extremities, induration surround central depression at PIP (donut sign) |
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What is the donut sign? |
Induration surrounding a central depressed area at the PIP in scleromyxedema |
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What are the systemic findings in scleromyxedema? |
Very frequently associated with visceral disease:
GI problems with dysphagia (MOST COMMON) Pulmonary complications Proximal muscle weakness CTS CNS complications (dermatoneuro syndrome- confusion, dizziness, dysarthria, sz) |
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Most common visceral complication associated wtih scleromyxedema? |
GI problems with dysphagia |
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Scleromyxedema has frequently been associated with which disease? |
Hep C
Monoclonal gammopathy (lambda) |
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Criteria for scleromyxedema? |
Mucin deposition Fibroblast proliferation Normal thyroid tests Presence of monoclonal gammopathy (IgG lambda) |
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Break down of mucinoses: |
I. Mucinosus A. Primary 1. a/w thyroid a. myxedema b. pretibial myxedema 2. lichen myxedematosus a. localized (5) (1) discrete papular lichen myxedematosus (2) acral persistent papular mucinosis (3) self healing papular mucinosis (4) papular mucinosis of infancy (5) nodular lichen myxedematosus b. diffuse (1) (1) scleromyxedema (generalized lichen myxedematosus) c. atypical/intermediate (4) (1) scleredema (2) reticular erythematous mucinosis (3) digital mucous cyst (4) follicular mucinosis (pinkus) B. Secondary 1. CTD, GA, SCAR ect (associated finding) |
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Treatment for scleromyxedema? |
Treatment is aimed at the associated plasma cell dyscrasia (monogammopathy IgG lamda > kappa) --> chemo |
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Localized lichen myxedematosus aka papular mucinosis has 5 disease subsets.... what are they and why are they all in this category? |
NO GAMMOPATHY, NO VISCERAL INVOLVEMENT, NO THYROID DISEASE
1. Discrete papular lichen myxedematosus (a/w HIV infection) 2. Acral persistent papular mucinosis 3. Self healing (Juvenile) papular mucinosis (Juvenile = joints, can have polyarthritis, in adults, no arthralgias) 4. Papular mucinosis of infancy (onset during first months of life) 5. Nodular lichen myxedematosus |
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Atypical/intermediate lichen myxedematosus have 4 disease subsets.... what are they? |
1. Scleredema 2. Reticular erythematous mucinosis 3. Digital mucous cyst 4. Follicular mucinosis |
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This bro's HbA1c is 8.7%... |
Scleredema!!!
Stiffening and hardening of SQ tissue d/t increased collagen and mucin, most common on back, neck and shoulders |
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What are the clinical subtypes of scleredema? |
Scleredema of Bushke: 1. following streptococcal infection (resolves in 1-2 years) 2. a/w IDDM- typically does not improve with DM treatment 3. Non IDDM- more often a/w a monoclonal gammopathy (Scleredema of 'Bothske'- both IgG kappa > Lambda) **TQ |
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Monoclonal gammopathy with:
Scleredema Scleromyxedema Necrobiotic xanthogranuloma |
Scleredema: both IgG kappa > lambda (Scleredema of 'bothke')
Scleromyxedema: IgG lambda
NXG: IgG kappa (bad kappa bacon) |
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Which type of atypical/intermediate mucinosis? |
Reticular erythematous mucinosis
erythematous plaques on mid chest, usually in young/middle aged females, OCP/pregnancy can worsen |
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Follicular mucinosis can be associated with what distressing finding? |
alopecia!!
may be skin limited and benign, or the beginning of CTCL (as everything else in derm :/) |
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treatment? |
puncture and aspiration
digital mucous cyst |
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Pretibial myxedema is a classic manifestation of what underlying disease? |
hyperthyroidism and/or Graves disease |
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entrapment of what nerve can lead to foot drop? |
Pretibial thyroid dermopathy (formerly known as pretibial myxedema)
entrapment of peroneal nerves
bilatera, nontender, nonpitting nodules and plaques with variable coloring and waxy indurated appearance |
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Myxedema can be seen in hyper and hypothyroidism. Clinically how can you distinguish between them? |
You have to look at the surrounding information.
Hyperthyroidism: overwhelming majority of patients also have exophthalmos
Hypothyroidism: skin is round and dry, puffy face, diffuse hair loss, lateral 1/3 of eyebrows lost |
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LOOK AT THE CASES FROM LECTURE |
DO IT NOW! |
